Improvement of cholestatic episodes in patients with benign recurrent intrahepatic cholestasis (BRIC) treated with rifampicin. A long-term follow-up.

OBJECTIVE: Patients with benign recurrent intrahepatic cholestasis (BRIC) suffer from recurrent episodes of cholestatic jaundice. Treatment options remain limited and are mainly symptomatic. In case reports rifampicin, plasmapheresis, and nasobiliary drainage have been reported to be effective. In this case series, we present long-term experience indicating disease-modifying effects of non-invasive treatment with rifampicin for recurrent cholestasis in BRIC type 1 (BRIC1). MATERIALS AND METHODS: We included all adult BRIC1 patients diagnosed and followed up at a single centre in Bergen, Norway. Data regarding clinical and biochemical features during BRIC attacks with and without rifampicin treatment were retrieved from medical journals and a data registry. RESULTS: Five males with BRIC1 were included. Median age at diagnosis was 22 years (range 15-41). Together they had suffered from 65 cholestatic attacks (including four documented abortive attacks). Twenty-eight attacks were treated with rifampicin alone over the last 12 years; all cases showed symptomatic relief and reduction in the levels of bilirubin and alkaline phosphatase in blood. The attacks treated with rifampicin seemed to have shorter duration and were less likely to result in complications or hospitalization compared to attacks prior to the introduction of rifampicin. No side effects attributable to rifampicin were noted. CONCLUSIONS: Episodic treatment of recurrent BRIC1 attacks with rifampicin seems to ameliorate severity and shorten the duration of attacks. Timely diagnosis and effective treatment are of major importance in BRIC, not only to decrease complications but also improving patients' quality of life.

Drug-induced esophageal injuries with an atypical presentation mimicking acute coronary syndrome.

BACKGROUND: Pill-induced esophageal injury may cause severe complications if not diagnosed in a timely fashion. The condition is under-recognized and under-reported, and some patients present with atypical clinical or endoscopic features mimicking other common conditions. If the diagnosis is missed the patient will continue to take the offending drug, potentially worsening the illness. We present a case in which acute coronary syndrome was the initial working diagnosis leading to a delay in diagnosis of doxycycline-induced esophageal injury. The patient developed multiple esophageal ulcers and hemorrhage. CASE PRESENTATION: A 50-year-old male driver with a history of hypertension and dyslipidemia was brought to the emergency department with complaints of severe retrosternal chest pain, vomiting, diaphoresis and syncope. On initial evaluation, acute coronary syndrome was considered due to the clinical presentation and history of cardiovascular risk factors. Electrocardiogram and serum troponins were normal. On the second day of his admission, the patient developed odynophagia and bloody vomitus. Esophagogastroduodenoscopy revealed extensive esophageal ulcerations with hemorrhage. The patient was taking Doxycycline capsules for an acute febrile illness. Doxycycline is the oral medication most commonly reported to cause esophageal injury. Doxycycline was discontinued, and the patient was treated with intravenous omeprazole and oral antacid suspension. The patient improved, was discharged after 6 days of hospitalization, and reported resolution of all symptoms at an outpatient follow-up visit 3 weeks later. CONCLUSION: Medication-induced esophageal injury can present with atypical symptoms mimicking acute coronary syndrome. This condition should be included in the initial differential diagnosis of patients presenting with acute chest pain, especially those taking oral medications known to cause esophageal injury.

Benign recurrent intrahepatic cholestasis: review and long-term follow-up of five cases.

OBJECTIVE: Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive inherited disorder characterized by intermittent episodes of cholestatic jaundice. For the patients, the disease is a physical and psychological challenge. There is no curable treatment, but symptomatic relief is described following treatment with rifampicin or plasmapheresis. MATERIAL AND METHODS: Five patients suffering from BRIC followed up for 17 years by one consultant are described. Two patients were treated with rifampicin and plasmapheresis, two with rifampicin alone, and one with plasmapheresis. RESULTS: The treatments showed symptomatic relief, effect on biochemical parameters, and earlier clinical remission compared with no treatment or treatment with other substances like cholestyramine, antihistamines, and ursodeoxycholic acid. CONCLUSIONS: Both rifampicin and plasmapheresis represent important therapeutic options of acute cholestatic attacks in patients with BRIC. As a noninvasive treatment, rifampicin may be the first choice.

Sonography of the small intestine.

In the last two decades, there has been substantial development in the diagnostic possibilities for examining the small intestine. Compared with computerized tomography, magnetic resonance imaging, capsule endoscopy and double-balloon endoscopy, ultrasonography has the advantage of being cheap, portable, flexible and user- and patient-friendly, while at the same time providing the clinician with image data of high temporal and spatial resolution. The method has limitations with penetration in obesity and with intestinal air impairing image quality. The flexibility ultrasonography offers the examiner also implies that a systematic approach during scanning is needed. This paper reviews the basic scanning techniques and new modalities such as contrast-enhanced ultrasound, elastography, strain rate imaging, hydrosonography, allergosonography, endoscopic sonography and nutritional imaging, and the literature on disease-specific findings in the small intestine. Some of these methods have shown clinical benefit, while others are under research and development to establish their role in the diagnostic repertoire. However, along with improved overall image quality of new ultrasound scanners, these methods have enabled more anatomical and physiological changes in the small intestine to be observed. Accordingly, ultrasound of the small intestine is an attractive clinical tool to study patients with a range of diseases.

High-dose ursodeoxycholic acid in primary sclerosing cholangitis: a 5-year multicenter, randomized, controlled study.

BACKGROUND & AIMS: There is no medical treatment of proven benefit for primary sclerosing cholangitis. This study aimed at studying the effect of a higher dose of ursodeoxycholic acid than previously used on survival, symptoms, biochemistry, and quality of life in this disease. METHODS: A randomized placebo-controlled study was performed in tertiary and secondary gastroenterology units. A total of 219 patients were randomized to 17 to 23 mg/kg body weight per day of ursodeoxycholic acid (n = 110) or placebo (n = 109) for 5 years. Follow-up data are available from 97 patients randomized to ursodeoxycholic acid and for 101 randomized to placebo. Quality of life was assessed by using the Medical Outcomes Study 36-item Short-Form Health Survey. RESULTS: The combined end point "death or liver transplantation" occurred in 7 of 97 (7.2%) patients in the ursodeoxycholic acid group vs 11 of 101 (10.9%) patients in the placebo group (P = .368; 95% confidence interval, -12.2% to 4.7%). The occurrence of liver transplantation as a single end point showed a similar positive trend for ursodeoxycholic acid treatment (5/97 [5.2%] vs 8/101 [7.9%]; 95% confidence interval, -10.4% to 4.6%). Three ursodeoxycholic acid and 4 placebo patients died from cholangiocarcinoma, and 1 placebo patient died from liver failure. Alkaline phosphatase and alanine aminotransferase tended to decrease during the first 6 months. There were no differences between the 2 groups in symptoms or quality of life. Analyses of serum ursodeoxycholic acid concentration gave no evidence that noncompliance may have influenced the results. CONCLUSIONS: This study found no statistically significant beneficial effect of a higher dose of ursodeoxycholic acid than previously used on survival or prevention of cholangiocarcinoma in primary sclerosing cholangitis.